Journal of Scleroderma and Related Disorders, Volume 2, Issue 3, September-December 2017, Pages 221-224

© 2017 SAGE Publications

, Article Reuse Guidelines

https://doi.org/10.5301/jsrd.5000257

Brief Report

Ulnar artery vasculopathy: a common but nonspecific feature of systemic sclerosis

Tae-Jong Kim¹, Soon-Young Song², Hee Chang Ahn³, Yoon-Kyoung Sung⁴, Sang-Cheol Bae⁴, and Jae-Bum Jun⁴

Abstract

Introduction

Systemic sclerosis (SSc) is characterized by microvascular abnormalities and fibrosis. Several studies have reported that large vessel disease is also common in SSc. The aim of this case series was to investigate whether ulnar artery involvement in patients with SSc is a disease-specific phenomenon, as compared to other connective tissue diseases (CTD).

Methods

A total of 28 patients, including 7 with SSc and 12 with systemic lupus erythematosus (SLE), underwent brachial arteriography due to severe Raynaud's phenomenon and/or digital ulcerations and were enrolled in the study. They were divided into two groups: an SSc/SSc-overlap group and a non-SSc group. The collection of the clinical parameters was conducted to investigate the associations between clinical factors and the ulnar artery vasculopathy.

Results

The SSc/SSc-overlap group (n = 10) consisted of 7 patients with SSc and 3 with features overlapping SSc. In the non-SSc group (n = 18), 12 cases of SLE, 2 of mixed connective tissue disease, 1 of dermatomyositis + SLE, 1 of rheumatoid arthritis, 1 of Sjogren's syndrome, and 1 case of skin vasculitis, were included. The relative frequencies of ulnar artery involvement were not significantly different between the SSc/SSc-overlap and non-SSc groups, respectively (n = 6, 60% vs. n = 9, 50%, p = 0.611).

Conclusions

Although ulnar artery involvement was frequently detected in patients with SSc/SSc-overlap, it was also a common finding in other CTDs; therefore, it is not specific to SSc or SSc-overlap diseases.

Keywords

Connective tissue diseases, Systemic sclerosis, Ulnar artery, Vasculopathy

¹ Department of Rheumatology, Chonnam National University Medical School and Hospital, Gwangju - Republic of Korea

² Department of Radiology, Hanyang University Hospital for Rheumatic Diseases, Seoul - Republic of Korea

³ Department of Plastic and Reconstructive Surgery, Hanyang University Seoul Hospital, Seoul - Republic of Korea

⁴ Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Seoul - Republic of Korea

Corresponding author(s):

Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, 222-1 Wangshimni-ro, Seongdong-gu, 04763 Seoul, Republic of Korea, junjb@hanyang.ac.kr

Introduction

Systemic sclerosis (SSc) is an autoimmune connective tissue disease (CTD) characterized by microvascular abnormalities and tissue fibrosis (1). Recently, several studies have reported that macrovascular involvement is a common feature of SSc (2–3–4). Involvement of the ulnar artery is far more common than involvement of the radial artery. This is important because the ulnar artery is presumed to be the predominant artery providing blood flow to the hand (5). Furthermore, an increased prevalence of ulnar artery occlusion in patients with SSc was found to be associated with severe refractory digital ulceration (6, 7). The aim of this study was to examine whether ulnar artery vasculopathy is specific to SSc. We analyzed the results of brachial arteriograms, which have been performed in several rheumatic diseases, including SSc.

Methods

Twenty-eight patients with CTDs who underwent brachial arteriography to evaluate severe Raynaud's phenomenon and/or digital ulcer/gangrene between May 2007 and June 2015 were enrolled. The diagnosis of CTD was based on the American College of Rheumatology criteria for rheumatoid arthritis (RA) (8), systemic lupus erythematosus (SLE) (9), SSc (10), and polyarteritis nodosa (PAN) (11). Two patients with mixed connective tissue disease (MCTD) met the Alarcon-Segovia criteria (12). Dermatomyositis (DM) was based on the Bohan criteria (13). Sjogren's syndrome (SS) and skin vasculitis were diagnosed by clinical, laboratory, and pathologic findings.

Clinical and laboratory data were collected via medical record review and included age, gender, disease duration, autoantibody profiles, pulmonary function test results, and the presence of complications such as interstitial lung diseases and pulmonary arterial hypertension.

The brachial arteriography images were obtained as previously described (4). The results were reviewed by an experienced angiographic radiologist (Song SY) for radial and ulnar artery occlusion or stenosis. The patency of their branches, including the deep palmar arch (DPA), superficial palmar arch (SPA), princeps pollicis artery (PPA), common palmar digital artery, and the proper palmar digital artery, were also assessed.

We divided the patients into two groups: SSc/SSc-overlap group and non-SSc rheumatic disease group. The frequency of ulnar artery involvement (occlusion and/or stenosis) including other clinical manifestations was investigated in the two groups with a chi-square or Fisher's exact test. Clinical comparisons were performed using t-tests for normally distributed continuous variables, and the Mann-Whitney rank-sum test was used for continuous variables that were not normally distributed. All p values <0.05 were considered to be statistically significant.

Ethical approval for this study was obtained from the Institutional Review Board (IRB file No. 2015-07-022-001).

Results

The clinical characteristics of the 28 patients with SSc/SSc-overlap and non-SSc rheumatic diseases are summarized in Table I.

The SSc/SSc-overlap group consisted of 7 patients with SSc and 3 with SSc-overlapping disorders (2 SLE and 1 PAN). In the non-SSc group, 12 cases of SLE, 2 of MCTD, 1 of DM + SLE, 1 of RA, 1 of SS, and 1 case of skin vasculitis were included. All but one patient with SSc were female. The SSc/SSc-overlap group was older and had longer disease duration than the non-SSc group, but these were not statistically significant. The possible confounders such as smoking status, dyslipidemia, traditional risk factors for atherosclerosis were also described in Table I with no statistically significance.

The manifestations of severe Raynaud's phenomenon and/or digital ulcer, relevant medication at brachial arteriography, ulnar and radial artery involvement, and conducted therapeutic intervention were described in Table II.

Of the 28 patients, 15 had ulnar artery involvement. Meanwhile, only 4 patients had radial artery involvement. The findings of ulnar artery involvement were significantly higher compared to radial artery involvement in all CTD groups investigated (53.6% vs. 14.3%, p = 0.002). However, the frequency of ulnar artery involvement was not significantly different in the SSc/SSc-overlap group compared to the non-SSc group (60% vs. 50%, p = 0.611). This was also the case upon comparison of individuals with pure SSc (n = 7) and pure SLE (n = 12). There was no difference in the frequency of ulnar artery involvement (occlusive lesions) between both groups, even if only occlusion was considered (5 cases in SSc group and 6 cases in non-SSc group) except for stenotic lesion (1 case in SSc group and in 3 cases non-SSc groups), because the degree of stenosis and its hemodynamic consequences are not known exactly.

The frequency of radial artery, SPA, and DPA involvement did not differ significantly between the two groups.

Angiographic findings are shown in detail in Supplementary Table I available online as Supplementary Material at www.sclerodermajournal.com. Of the 28 patients, 15 had ulnar artery involvement (7 SLE, 4 SSc, 1 SLE + DM, 1 SSc + PAN, 1 RA, 1 SSc + SLE). Among these 15 patients, 6 cases were bilateral and 9 were unilateral. Involvement type was occlusive in 11 patients and stenotic in 4 patients.

Only four patients had radial artery involvement (2 SSc, 1 SLE, 1 RA), with no statistical difference between the two groups. Interestingly, all patients with radial artery involvement also had ulnar artery involvement. In other words, isolated involvement of the radial artery did not exist in these patients.

Discussion

In this study, we demonstrated that ulnar artery vasculopathy was common in, but not specific to, SSc. We could not explain why occlusion or stenosis of the ulnar artery was more common than that of the radial artery in SSc and the other rheumatic diseases. The predominance of ulnar artery involvement seems to be clinically critical, since it is thought to provide the main blood supply to the hand (5), although this has not been definitively shown. Still, ulnar artery occlusion is reported to be predictive of digital ulceration in SSc (14), which supports the afore-mentioned hypothesis.

The relative size of the ulnar compared to the radial artery may explain the predominant involvement of the ulnar artery. Interestingly, however, there are no comprehensive reviews on the sizes of either artery. Recently, interventional cardiologists have been increasing their use of the radial artery for diagnostic and therapeutic purposes. This phenomenon precipitated the attention towards the size of the radial and ulnar arteries. To date, there is no consensus as to which artery is larger (15), but this seems to be dependent on the ethnic or regional origins of the source population. A systematic review regarding the relative sizes of the ulnar and radial arteries will be necessary.

This study compared only those patients with SSc and other rheumatic diseases who were planning vascular surgery, therefore was not nonselective.

In conclusion, although the population examined in the present study was small, we showed that ulnar artery vasculopathy is not specific to SSc, but rather common in a variety of rheumatic diseases, including SSc. Interestingly, isolated involvement of the radial artery did not exist in this study. Further studies are needed to identify this mechanism.

Funding

Financial support: No grants or funding have been received for this study.

Conflict of Interest

Conflict of interest: None of the authors has financial interest related to this study to disclose.

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Table I The general characteristics of patients between SSc/SSc-overlap and non-SSc rheumatic diseases

| SSc/SSc-overlap (n = 10) | Non-SSc (n = 18) | p
Diagnosis (n) | SSc (7), SSc + SLE (2), SSc + PAN (1) | SLE (12), MCTD (2), DM + SLE (1), RA (1), SS (1), Skin vasculitis (1) |
Female, n (%) | 9 (90) | 18 (100) | 0.357
Age, y, median (IQR) | 44 (39-54) | 39.5 (32-51) | 0.313
Duration, y, median (IQR) | 6.5 (5.3-14.3) | 6 (2-9) | 0.630
Smoking, n (present/tested) | 1/9 | 0/18 | 0.333
Dyslipidemia, n (present/tested) |  |  |
 HDL-C (M;<35 mg/dL, F;<40 mg/dL) | 5/6 | 9/14 | 0.613
 Triglyceride ≥150 mg/dL | 3/7 | 7/17 | 1.000
 LDL-C ≥130 mg/dL | 0/7 | 3/17 | 0.530
Autoantibody, n (present/tested) | Anti-topoisomerase I (2/8), anticentromere (1/6) | Anti-Sm (1/15), anti-DNA (6/16), anti-nRNP (9/14) | –
Morbidity, n (present/tested) | HT (1/8), diabetes (0/8), ILD (3/10), PH (1/5) | HT (1/18), diabetes (0/18), ILD (2/18), PH (2/10) | –
Cardiac involvement, n | Pericardial effusion (2), Heart failure (1) | Mitral valve prolapse with MR (1) | –

SSc = systemic sclerosis; SLE = systemic lupus erythematosus; PAN = polyarteritis nodosa; MCTD = mixed connective tissue disease; DM = dermatomyositis; RA = rheumatoid arthritis; SS = Sjogren syndrome; + = concomitant presence; IQR = interquartile range; HDL-C = high-density lipoprotein-cholesterol; M = male; F = female; LDL-C = low-density lipoprotein-cholesterol; HT = hypertension; ILD = interstitial lung disease by high-resonance computerized tomography; PH = pulmonary hypertension ≥50 mmHg of systolic pulmonary artery pressure by echocardiography; MR = mitral regurgitation.

Table II Digit status, relevant medication at angiography, vasculopathy, and therapeutic intervention in patients with rheumatic diseases with severe Raynaud's phenomenon and/or digital ulcer

Characteristics, n (present/tested) | SSc/SSc-overlap (n = 10) | Non-SSc (n = 18) | p
Digital status |  |  |
 DU or gangrene | 5/10 | 3/18 | 0.074
 Impending ischemia | 5/10 | 15/18 |
Medication at angiography |  |  |
 Vasodilator | 8/10 | 7/18 |
 Anti-platelet | 5/10 | 7/18 |
 PGE1 derivatives | 1/10 | 5/18 |
 Cilostazol | 1/10 | 0/18 | –
 Warfarin | 0/10 | 1/18 |
 No medication | 0/10 | 5/18 |
Vasculopathy |  |  |
 Ulnar artery vasculopathy | 6/10 | 9/18 | 0.705
  Occlusive lesion | 5/10 | 6/18 | 0.444
 Radial artery vasculopathy | 2/10 | 2/18 | 0.601
 SPA involvement | 8/10 | 11/18 | 0.417
 DPA involvement | 1/10 | 2/18 | 1.000
 Digital sympathectomy with or without reconstruction | 5/10 | 8/18 | 1.000

SSc = systemic sclerosis; DU = digital ulcer; PGE1 = prostaglandin E1; SPA = superficial palmar arch; DPA = deep palmar arch.